Lionel Ginsberg, Renzo Manara, Alan R Valentine, Brian Kendall, Alessandro P Burlina
{"title":"法布里病的磁共振成像变化。","authors":"Lionel Ginsberg, Renzo Manara, Alan R Valentine, Brian Kendall, Alessandro P Burlina","doi":"10.1111/j.1651-2227.2006.tb02391.x","DOIUrl":null,"url":null,"abstract":"<p><strong>Unlabelled: </strong>Recognized magnetic resonance imaging (MRI) abnormalities in the brains of patients with Fabry disease include the consequences of infarction and haemorrhage, non-specific white and grey matter lesions, vascular anomalies, in particular dolicho-ectasia, and a characteristic appearance of the posterior thalamus. A preliminary analysis of MRI findings in patients registered in FOS, the Fabry Outcome Survey, indicates that most patients had abnormal scans (25/47). The commonest abnormality, in males and females, was the presence of cerebral white matter lesions, the number of which increased with patient age.</p><p><strong>Conclusion: </strong>MRI is a valuable resource for assessing the CNS complications of Fabry disease, and their response to time and treatment.</p>","PeriodicalId":76973,"journal":{"name":"Acta paediatrica (Oslo, Norway : 1992). Supplement","volume":"95 451","pages":"57-62"},"PeriodicalIF":0.0000,"publicationDate":"2006-04-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":"0","resultStr":"{\"title\":\"Magnetic resonance imaging changes in Fabry disease.\",\"authors\":\"Lionel Ginsberg, Renzo Manara, Alan R Valentine, Brian Kendall, Alessandro P Burlina\",\"doi\":\"10.1111/j.1651-2227.2006.tb02391.x\",\"DOIUrl\":null,\"url\":null,\"abstract\":\"<p><strong>Unlabelled: </strong>Recognized magnetic resonance imaging (MRI) abnormalities in the brains of patients with Fabry disease include the consequences of infarction and haemorrhage, non-specific white and grey matter lesions, vascular anomalies, in particular dolicho-ectasia, and a characteristic appearance of the posterior thalamus. A preliminary analysis of MRI findings in patients registered in FOS, the Fabry Outcome Survey, indicates that most patients had abnormal scans (25/47). The commonest abnormality, in males and females, was the presence of cerebral white matter lesions, the number of which increased with patient age.</p><p><strong>Conclusion: </strong>MRI is a valuable resource for assessing the CNS complications of Fabry disease, and their response to time and treatment.</p>\",\"PeriodicalId\":76973,\"journal\":{\"name\":\"Acta paediatrica (Oslo, Norway : 1992). Supplement\",\"volume\":\"95 451\",\"pages\":\"57-62\"},\"PeriodicalIF\":0.0000,\"publicationDate\":\"2006-04-01\",\"publicationTypes\":\"Journal Article\",\"fieldsOfStudy\":null,\"isOpenAccess\":false,\"openAccessPdf\":\"\",\"citationCount\":\"0\",\"resultStr\":null,\"platform\":\"Semanticscholar\",\"paperid\":null,\"PeriodicalName\":\"Acta paediatrica (Oslo, Norway : 1992). Supplement\",\"FirstCategoryId\":\"1085\",\"ListUrlMain\":\"https://doi.org/10.1111/j.1651-2227.2006.tb02391.x\",\"RegionNum\":0,\"RegionCategory\":null,\"ArticlePicture\":[],\"TitleCN\":null,\"AbstractTextCN\":null,\"PMCID\":null,\"EPubDate\":\"\",\"PubModel\":\"\",\"JCR\":\"\",\"JCRName\":\"\",\"Score\":null,\"Total\":0}","platform":"Semanticscholar","paperid":null,"PeriodicalName":"Acta paediatrica (Oslo, Norway : 1992). Supplement","FirstCategoryId":"1085","ListUrlMain":"https://doi.org/10.1111/j.1651-2227.2006.tb02391.x","RegionNum":0,"RegionCategory":null,"ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":null,"EPubDate":"","PubModel":"","JCR":"","JCRName":"","Score":null,"Total":0}
Magnetic resonance imaging changes in Fabry disease.
Unlabelled: Recognized magnetic resonance imaging (MRI) abnormalities in the brains of patients with Fabry disease include the consequences of infarction and haemorrhage, non-specific white and grey matter lesions, vascular anomalies, in particular dolicho-ectasia, and a characteristic appearance of the posterior thalamus. A preliminary analysis of MRI findings in patients registered in FOS, the Fabry Outcome Survey, indicates that most patients had abnormal scans (25/47). The commonest abnormality, in males and females, was the presence of cerebral white matter lesions, the number of which increased with patient age.
Conclusion: MRI is a valuable resource for assessing the CNS complications of Fabry disease, and their response to time and treatment.